1. Current barriers and recommendations on the diagnosis of transthyretin amyloid cardiomyopathy: a Delphi study
- Author
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Yüksel Çavuşoğlu, İbrahim Başarıcı, Omaç Tüfekçioğlu, Ebru Özpelit, Elif Özdemir, İlknur Ak Sivrikoz, Hakan Altay, Muzaffer Değertekin, İrem Dinçer, Barış İkitimur, Gökhan Kahveci, Murat Fani Bozkurt, Metin Erkılıç, Gamze Çapa Kaya, Meral Beksaç, Ayşe Salihoğlu, and Lale Tokgözoğlu
- Subjects
amyloidosis ,transthyretin amyloid ,cardiomyopathy ,diagnosis ,cardiac scintigraphy ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
ObjectivesThis study has been conducted to investigate the non-invasive diagnostic journey of patients with a transthyretin amyloid cardiomyopathy (aTTR-CM) in Turkey, identify the challenges and uncertainties encountered on the path to diagnosis from the perspectives of expert physicians, and develop recommendations that can be applied in such cases.MethodsThis study employed a three-round modified Delphi method and included 10 cardiologists and five nuclear medicine specialists. Two hematologists also shared their expert opinions on the survey results related to hematological tests during a final face-to-face discussion. A consensus was reached when 80% or more of the panel members marked the “agree/strongly agree” or “disagree/strongly disagree” option.ResultsThe panelists unanimously agreed that the aTTR-CM diagnosis could be established through scintigraphy (using either 99mTc-PYP, 99mTc-DPD, or 99mTc-HMPD) in a patient with suspected cardiac amyloidosis (CA) without a further investigation if AL amyloidosis is ruled out (by sFLC, SPIE and UPIE). In addition, scintigraphy imaging performed by SPECT or SPECT-CT should reveal a myocardial uptake of Grade ≥2 with a heart-to-contralateral (H/CL) ratio of ≥1.5. The cardiology panelists recommended using cardiovascular magnetic resonance (CMR) and a detailed echocardiographic scoring as a last resort before considering an endomyocardial biopsy in patients with suspected CA whose scintigraphy results were discordant/inconclusive or negative but still carried a high clinical suspicion of aTTR-CM.ConclusionThe diagnostic approach for aTTR-CM should be customized based on the availability of diagnostic tools/methods in each expert clinic to achieve a timely and definitive diagnosis.
- Published
- 2024
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